Table 18.1 Nomenclature of bone tumors
(a) Pathology of cirrhosis. Histological appearance showing nodules of liver tissue of varying size surrounded by fibrosis.
(b) CT scan showing an irregular lobulated liver. There is splenomegaly and enlargement of collateral vessels beneath the anterior abdominal wall (arrows) as a result of portal hypertension.
(c) CT image showing cirrhosis, with a patent portal vein and no space-occupying lesion.
• Impotence, diabetes mellitus, hyperpigmentation, arthritis (hemochromatosis)
• Neurologic disturbances (Wilson’s disease, hepatolenticular degeneration)
• Family history of “liver disease” (hemochromatosis [positive family history in 28% of patients], a-1 antitrypsin deficiency)
• History of recurrent episodes of RUQ pain (biliary tract disease)
• History of blood transfusions, IV drug abuse (hepatitis C)
• History of hepatotoxic drug exposure
• Coexistence of other diseases with immune or autoimmune features (ITP, myasthenia gravis, thyroiditis, autoimmune hepatitis)
• Decreased Hgb and Hct, elevated MCV, increased BUN and creatinine (the BUN may also be “normal” or low if the patient has severely diminished liver function), decreased sodium (dilutional hyponatremia), decreased potassium (as a result of secondary aldosteronism or urinary losses)
• Decreased glucose in a patient with liver disease indicating severe liver damage
• Other laboratory abnormalities:
1. Alcoholic hepatitis and cirrhosis: there may be mild elevation of ALT and AST, usually <500 IU; AST > ALT (ratio >2:3).
2. Extrahepatic obstruction: there may be moderate elevations of ALT and AST to levels <500 IU.
3. Viral, toxic, or ischemic hepatitis: there are extreme elevations (>500 IU) of ALT and AST.
4. Transaminases may be normal despite significant liver disease in patients with jejunoileal bypass operations or hemochromatosis or after methotrexate administration.
5. Alkaline phosphatase elevation can occur with extrahepatic obstruction, primary biliary cirrhosis, and primary sclerosing cholangitis.
6. Serum LDH is significantly elevated in metastatic disease of the liver; lesser elevations are seen with hepatitis, cirrhosis, extrahepatic obstruction, and congestive hepatomegaly.
7. Serum glutamyl transpeptidase (GGTP) is elevated in alcoholic liver disease and may also be elevated with cholestatic disease (primary biliary cirrhosis, primary sclerosing cholangitis).
8. Serum bilirubin may be elevated; urinary bilirubin can be present in hepatitis, hepatocellular jaundice, and biliary obstruction.
9. Serum albumin: significant liver disease results in hypoalbuminemia.
10. Prothrombin time: an elevated PT in patients with liver disease indicates severe liver damage and poor prognosis.
11. Presence of hepatitis B surface antigen implies acute or chronic active hepatitis B.
12. Presence of antimitochondrial antibody suggests primary biliary cirrhosis, chronic active hepatitis.
13. Elevated serum copper, decreased serum ceruloplasmin, and elevated 24-hours urine may be diagnostic of Wilson’s disease.
14. Protein immunoelectrophoresis may reveal decreased a-1 globulins (a-1 antitrypsin deficiency), increased IgA (alcoholic cirrhosis), increased IgM (primary biliary cirrhosis), increased IgG (chronic active hepatitis, cryptogenic cirrhosis).
15. An elevated serum ferritin and increased transferrin saturation are suggestive of hemochromatosis.
16. An elevated blood ammonia suggests hepatocellular dysfunction; serial values are not useful in following patients with hepatic encephalopathy because there is poor correlation between blood ammonia level and degree of hepatic encephalopathy.
17. Serum cholesterol is elevated in cholestatic disorders.
18. Antinuclear antibodies (ANA) may be found in autoimmune hepatitis.
19. Alpha fetoprotein: levels >1000 pg/ml are highly suggestive of primary liver cell carcinoma.
20. Anti-hepatitis C virus identifies patient with prior hepatitis C virus infection.
21. Elevated level of serum globulin (especially a-globulins) may occur with autoimmune hepatitis.
• Ultrasonography is the procedure of choice for detection of gallstones and dilation of common bile ducts.
• CT scan is useful for detecting mass lesions in liver and pancreas, assessing hepatic fat content, identifying idiopathic hemochromatosis, early diagnosing of Budd-Chiari syndrome, dilation of intrahepatic bile ducts, and detection of varices and splenomegaly.
• Technetium-99m sulfur colloid scanning is useful for diagnosing cirrhosis (there is a shift of colloid uptake to the spleen, bone marrow), identifying hepatic adenomas (cold defect is noted), diagnosing Budd-Chiari syndrome.
• ERCP is the procedure of choice for diagnosing periampullary carcinoma, common duct stones; it is also useful in diagnosing primary sclerosing cholangitis.
• Percutaneous transhepatic cholangiography (PTC) is useful when evaluating patients with cholestatic jaundice and dilated intrahepatic ducts by ultrasonography; presence of intrahepatic strictures and focal dilation is suggestive of PSC.
• Percutaneous liver biopsy is useful inevaluating hepatic filling defects, diagnosing hepatocellular disease or hepatomegaly, evaluating persistently abnormal liver function tests, and diagnosing hemachromatosis, primary biliary cirrhosis, Wilson’s disease, glycogen storage diseases, chronic hepatitis, autoimmune hepatitis, infiltrative diseases, alcoholic liver disease, drug-induced liver disease, and primary or secondary carcinoma.
Avoid any hepatotoxins (e.g., ethanol, acetaminophen); improve nutritional status.
ACUTE GENERAL Rx
• Correct any mechanical obstruction to bile flow (e.g., calculi, strictures).
• Provide therapy for underlying cardiovascular disorders in patients with cardiac cirrhosis.
• Remove excess body iron with phlebotomy and deferoxamine in patients with hemochromatosis.
• Remove copper deposits with D-penicillamine in patients with Wilson’s disease.
• Long-term ursodiol therapy will slow the progression of primary biliary cirrhosis. It is, however, ineffective in primary sclerosing cholangitis.
• Glucocorticoids (prednisone 20 to 30 mg/day initially or combination therapy or prednisone and azathioprine) is useful in autoimmune hepatitis.
• Liver transplantation may be indicated in otherwise healthy patients (age <65 years) with sclerosing cholangitis, chronic hepatitis cirrhosis, or primary biliary cirrhosis with prognostic information suggesting < 22% chance of survival without transplantation; contraindications to liver transplantation are AIDS, most metastatic malignancies, active substance abuse, uncontrolled sepsis, uncontrolled cardiac or pulmonary disease.
• Treatment of complications of portal hypertension (ascites, esophagogastric varices, hepatic encephalopathy, and hepatorenal syndrome)
• Prognosis varies with the etiology of the patient’s cirrhosis and whether there is ongoing hepatic injury. Mortality rate exceeds 80% in patients with hepatorenal syndrome.
• Two markers of portal hypertension, thrombocytopenia and splenomegaly, moderately increase the likelihood of large esophageal varices.
• If advanced cirrhosis is present and transplantation is not feasible, survival is 1 to 2 years.
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DON'T FORGET - KNOWLEDGE IS EVERYTHING!
Cirrhosis is defined histologically as the presence of fibrosis and regenerative nodules in the liver. It can be classified as micronodular, macronodular, and mixed; however, each form may be seen in the same patient at different stages of the disease. Cirrhosis manifests clinically with portal hypertension, hepatic encephalopathy, and variceal bleeding.
EPIDEMIOLOGY & DEMOGRAPHICS
• Cirrhosis is the eleventh leading cause of death in the USA (death rate 11 deaths / 100,000 persons / year).
• Alcohol abuse and viral hepatitis are the major causes of cirrhosis in the USA.
PHYSICAL FINDINGS & CLINICAL PRESENTATION
SKIN: Jaundice, palmar erythema (alcohol abuse), spider angiomata, ecchymosis (thrombocytopenia or coagulation factor deficiency), dilated superficial periumbilical vein (caput medusae), increased pigmentation (hemochromatosis), xanthomas (primary biliary cirrhosis), needle tracks (viral hepatitis)
EYES: Kayser-Fleischer rings (corneal copper deposition seen in Wilson’s disease; best diagnosed with slit lamp examination), scleral icterus
BREATH: Fetor hepaticus (musty odor of breath and urine found in cirrhosis with hepatic failure)
CHEST: Possible gynecomastia in men
ABDOMEN: Tender hepatomegaly (congestive hepatomegaly), small, nodular liver (cirrhosis), palpable, nontender gallbladder (neoplastic extrahepatic biliary obstruction), palpable spleen (portal hypertension), venous hum auscultated over periumbilical veins (portal hypertension), ascites (portal hypertension, hypoalbuminemia)
RECTAL EXAMINATION: Hemorrhoids (portal hypertension), guaiac-positive stools (alcoholic gastritis, bleeding esophageal varices, PUD, bleeding hemorrhoids)
GENITALIA: Testicular atrophy in males (chronic liver disease, hemochromatosis)
EXTREMITIES: Pedal edema (hypoalbuminemia, failure of right side of the heart), arthropathy (hemochromatosis)
NEUROLOGIC: Flapping tremor, asterixis (hepatic encephalopathy), choreoathetosis, dysarthria (Wilson’s disease)
• Alcohol abuse
• Secondary biliary cirrhosis, obstruction of the common bile duct (stone, stricture, pancreatitis, neoplasm, sclerosing cholangitis)
• Drugs (e.g., acetaminophen, isoniazid, methotrexate, methyldopa)
• Hepatic congestion (e.g., CHF, constrictive pericarditis, tricuspid insufficiency, thrombosis of the hepatic vein, obstruction of the vena cava)
• Primary biliary cirrhosis
• Chronic active hepatitis caused by hepatitis B or C
• Wilson’s disease
• a-1 antitrypsin deficiency
• Infiltrative diseases (amyloidosis, glycogen storage diseases, hemochromatosis)
• Nutritional: jejunoileal bypass
• Others: parasitic infections (schistosomiasis), idiopathic portal hypertension, congenital hepatic fibrosis, systemic mastocytosis, autoimmune hepatitis, hepatic steatosis, IBD.
The characteristic features of cirrhosis are regenerating nodules separated by fibrous septa and loss of the normal lobular architecture within the nodules (Fig. 7.20 a). Two types of cirrhosis have been described which give clues to the underlying cause:
■ Micronodular cirrhosis. Regenerating nodules are usually less than 3 mm in size and the liver is involved uniformly. This type is often caused by ongoing alcohol damage or biliary tract disease.
■ Macronodular cirrhosis. The nodules are of variable size and normal acini may be seen within the larger nodules. This type is often seen following chronic viral hepatitis.
Diagnostic workup is aimed at identifying the most likely cause of cirrhosis. The history is extremely important:
• Alcohol abuse: alcoholic liver disease
• History of hepatitis B (chronic active hepatitis, primary hepatic neoplasm, or hepatitis C)
• History of IBD (primary sclerosing cholangitis)
• History of pruritus, hyperlipoproteinemia, and xanthomas in a middle-aged or elderly female (primary biliary cirrhosis)